PReS-FINAL-2173: Protein kinase C delta deficiency is a new cause of monogenic SLE

Systemic lupus erythematosus (SLE) is a prototype autoimmune disease. Infectious triggers, genetic background, immunological abnormalities and environmental factors are all supposed to interact in disease development. Rare causes of monogenic SLE have been described, (e.g. complement deficiencies, interferonopathies and FasL deficiency) providing unique insights into fundamental mechanisms of immune tolerance.